Livedo vasculopathy associated with methylenetetrahydrofolate reductase C677T homozygosity

and has a very progressive malignant behavior with high rate of lymph node metastases. Therefore, early diagnosis and immediate treatment is rather important. We suggest repeated biopsies should be performed in such suspected patients. However, fine-needle aspiration cytology is not recommended because tumor cells may be heterogeneous and sporadic in a mass rather than equally and extensively distributed under microscope. Treatment options include surgical excision, radiotherapy, chemotherapy and intralesional cytokine injection. The reason for this successful treatment was probably on one hand the fact that histopathology showed a welldifferentiated variety of tumor without distant metastasis, and on the other hand wide excision was performed to the best of our ability. What the inner link is between the coexistence of PH and angiosarcoma is uncertain. Pemphigus accompanied with angiosarcoma has not been reported in human beings although a horse case was reported in the 20th century. The authors believed that the bullous stomatitis in that horse was a mucocutaneous form of paraneoplastic syndrome because signs ceased shortly after tumor removal. We do not deem that there was an inevitable etiological association between PH and angiosarcoma in this case; otherwise, the PH could not have been easily controlled within 2 weeks with a low dose of corticosteroids before surgical excision of the tumor. On the contrary, drug-induced PH could not be excluded because pemphigus has sometimes been a variant of drug reaction.